ABSTRACT
@#Prurigo pigmentosa is an inflammatory dermatosis characterized by a pruritic, symmetrically distributed erythematous papular or papulo-vesicular eruption on the trunk arranged in a reticulated pattern that resolves with hyperpigmentation. It is typically non-responsive to topical or systemic steroid therapy. The exact etiology is unknown, but it is more commonly described in the Far East countries. Dietary change is one of the predisposing factors. We report on nine young adult patients with prurigo pigmentosa, among whom five were on ketogenic diets prior to the onset of the eruptions. All cases resolved with oral doxycycline with no recurrence. We hope to improve the awareness of this uncommon skin condition among general practitioners and physicians so that disfiguring hyperpigmentation due to delayed diagnosis and treatment can be avoided.
ABSTRACT
BACKGROUND: Prurigo pigmentosa (PP) is a chronic recurrent dermatosis that occurs in young adults and is characterized by pruritic erythematous macules and papules that leave reticular hyperpigmentation. Although there have been a few comprehensive studies, the clinical characteristics and recurrence rates have not been fully elucidated. OBJECTIVE: The purpose of this study was to evaluate the clinical findings and recurrence rates according to the factors associated with PP. METHODS: We reviewed the medical records, photographs, and histopathological slides of 67 patients who were diagnosed with PP between March 2005 and March 2013. Statistical analysis was performed to compare the recurrence rates between the group without associated factors and the group with associated factors (contact, diet/weight loss, and sweat, respectively). RESULTS: PP associated with contact showed localized involvement and transverse direction of the lesions; however, the PP lesions associated with diet or weight loss showed diffuse involvement. PP associated with sweat showed a tendency to be distributed in the flexural area and depressed area with longitudinal direction of the lesions. The recurrence rate of the group with no associated factors was 45.7%, while that of the group with associated factors was 33.3%. Within this group, the recurrence rate was 23.1% (3/13) for the contact group, 27.3% (3/11) for the diet/weight loss group, and 50% (4/8) for the sweat group. However, the differences in recurrence rates were not statistically significant. CONCLUSION: We suspect that there are differences in the clinical characteristics according to the factors associated with PP. Although the differences in recurrence rates according to the factors associated with PP were not statistically significant, we suspect that further research with a larger sample size will show that the differences are in fact present.
Subject(s)
Humans , Young Adult , Diet , Hyperpigmentation , Medical Records , Prurigo , Recurrence , Sample Size , Skin Diseases , Sweat , Weight LossABSTRACT
BACKGROUND: Prurigo pigmentosa is a rare recurrent inflammatory disease of the skin, and this is characterized by an abrupt onset of erythematous papules that usually resolve with leaving reticular hyperpigmentation. Although its incidence has increased in Korea as well as in the international community, there are few comprehensive studies on this malady in the Korean dermatologic literature. OBJECTIVE: The purpose of this study was to evaluate the clinical and histopathological features of prurigo pigmentosa. METHODS: We reviewed all the medical records, photographs and histopathological slides of 27 patients who had been diagnosed with prurigo pigmentosa at 3 hospitals between 1998 and 2009. Additionally, we also reviewed the clinical findings of the 37 cases that have been reported in the Korean dermatologic literature. RESULTS: The ratio of males to females was 1:2.4 and the mean age of onset was 24.6+/-8.2 years old. Twenty six patients (96.3%) complained of pruritus that was usually moderate to severe and the most commonly involved site was the back (63.0%). Although all 27 patients presented with erythematous papules, the skin lesions were accompanied with reticulated hyperpigmentation in 17 patients (63.0%) at the time of diagnosis. Fourteen patients were treated with minocycline alone or in combination therapy, and two pregnant women were treated with topical steroid alone. The major microscopic features of the lesions of prurigo pigmentosa were mild to moderate acanthosis and spongiosis in the epidermis, extravasation of erythrocytes and a mild to moderate perivascular lymphohistiocytic infiltrate in the dermis. A neutrophilic infiltrate tended to be observed in the relatively early lesions rather than in the fully developed lesions. CONCLUSION: Clinicopathologic correlation and thorough long-term follow-up are necessary to establish a diagnosis of prurigo pigmentosa because a pathologic examination does not show pathognomonic features. Our data suggest that neutrophils may play a role in the pathogenesis of prurigo pigmentosa, based upon finding them in the early lesion and the efficacy of the agents that inhibit neutrophil function, such as dapsone or minocycline.
Subject(s)
Female , Humans , Male , Age of Onset , Dapsone , Dermis , Epidermis , Erythrocytes , Follow-Up Studies , Hyperpigmentation , Incidence , Korea , Medical Records , Minocycline , Neutrophils , Pregnant Women , Prurigo , Pruritus , SkinABSTRACT
Prurigo pigmentosa (PP) is a rare inflammatory skin disease of unknown etiology, characterized by recurrent, symmetrical, pruritic, erythematous papules resulting in gross reticular hyperpigmentation. The rash occurs mainly on the back, the chest and the nape of the neck. Vesicular or bullous forms have been reported only rarely. This case report concerns a 15-year-old female with PP, in whom vesicular manifestations appeared recurrently on an existing maculopapular eruption on the trunk.
Subject(s)
Adolescent , Female , Humans , Blister , Exanthema , Hyperpigmentation , Neck , Prurigo , Skin Diseases , ThoraxABSTRACT
Prurigo pigmentosa is a rare dermatosis characterized by the sudden onset of pruritic erythematous papules that leave reticulated, mottled hyperpigmentation after healing. Prurigo pigmentosa is an unique inflammatory dermatosis that has distinctive phases of clinical and histopathological features. The cause is unknown. Skin lesions are symmetrically distributed on the neck, clavicular, chest and upper back area in young adults, predominantly women. This disease is endemically common in Japan but rarely seen in other countries (especially western). In Korean dermatologic literature, there are some reported cases of prurigo pigmentosa. We report the cases of 5 patients who were diagnosed as prurigo pigmentosa.
Subject(s)
Female , Humans , Young Adult , Hyperpigmentation , Japan , Neck , Prurigo , Skin , Skin Diseases , ThoraxABSTRACT
Prurigo pigmentosa is a rare and recurrent inflammatory dermatosis characterized clinically by severe pruritic erythematous papules and reticulated pigmentation symmetrically distributed on the trunk and neck in young adolescent females. The pathogenesis remains unclear but environmental factors such as friction from the clothing and para-amino compounds used in manufacturing of clothing have been suspected. We report a case of prurigo pigmentosa that was supposedly relevant to the swimming suit and treated with dapsone and minocycline resulting in significant clinical improvement.
Subject(s)
Adolescent , Female , Humans , Clothing , Dapsone , Friction , Minocycline , Neck , Pigmentation , Prurigo , Skin Diseases , SwimmingABSTRACT
Prurigo pigmentosa is a rare inflammatory dermatosis of unknown etiology characterized by recurrent, pruritic, erythematous papules with gross reticulate hyperpigmentation. The cause of prurigo pigmentosa is unknown. Exogenous factors, fasting, dieting, ketosis, diabetes mellitus and pregnancy have been reported to be associated with prurigo pigmentosa. We now present two cases of prurigo pigmentosa associated with dieting. These findings suggest that the ketosis produced by dieting may well contribute to the pathogenesis of prurigo pigmentosa.
Subject(s)
Pregnancy , Diabetes Mellitus , Diet , Fasting , Hyperpigmentation , Ketosis , Prurigo , Skin DiseasesABSTRACT
Prurigo pigmentosa - a rare dermatosis described first by Nagashima in 1971 - presents as intensely pruritic papules arranged in a reticular pattern and distributed symmetrically on the back of young women. Individual lesions involute within weeks, leaving net-like pigmentation, but recurrences are the rule. According to the recent report of Ackerman et al., prurigo pigmentosa shows discernable characteristic histopathologic stages successively in its natural course. We report two cases of prurigo pigmentosa with clinico-histopathologic analysis - one, occurred in 24-year old man who recovered spontaneously after 7 months of morbidity and the other, occurred in 23-year old woman who responded dramatically to dapsone.
Subject(s)
Female , Male , HumansABSTRACT
Prurigo pigmentosa is a chronic inflammatory dermatosis characterized by reticulated erythematous papules, hyperpigmentation with severe pruritus and usually occurs in young femals around their twenties. Most cases have been reported from Japan, but only twelve cases have been reported in Korea. Histopathologic findings of erythematous papules shows spongiosis, exocytosis, liquefaction degeneration of the basal cell, perivascular lymphohistiocytic infiltration in the dermis and finally dermal fibrosis and deposit of dermal melanophages in hyperpigmented lesions. We report three cases of prurigo pigmentosa which showed various histopathologic features of early, fully-developed and late stage, respectively.
Subject(s)
Dermis , Exocytosis , Fibrosis , Hyperpigmentation , Japan , Korea , Prurigo , Pruritus , Skin DiseasesABSTRACT
Prurigo pigmentosa is a rare dermatosis with unknown etiology. It is characterized by the sudden development of erythematous papules that resolve leaving reticulated hyperpigmentation mainly on the trunk. Nearly 300 cases have been reported in Japan to date. A 17-year-old male came to our hospital with a 2-month history of pruritic eruption on the left clavicular area, back and nape. Before he visited our hospital, he was treated with topical corticosteroids and oral antihistamine but the eruption and the pruritus continued. We treated him with doxycycline, 200mg daily. Two weeks later, leaving brownish reticular pigmentation, the erythema and the pruritus disappeared. Over a year there has been no recurrence.
Subject(s)
Adolescent , Humans , Male , Adrenal Cortex Hormones , Doxycycline , Erythema , Hyperpigmentation , Japan , Pigmentation , Prurigo , Pruritus , Recurrence , Skin DiseasesABSTRACT
Prurigo pigmentosa is a papular pruriginous eruption of unknown origin that leaves a reticulated, mottled hyperpigmentation. The condition is rare in the Western world, but many cases have been described in Japan. In Korea, six cases of prurigo pigmentosa have been reported, but follow-up of clinical course from the onset to the late stage was not performed. We report a case of prurigo pigmentosa, followed-up for 4 years, in a 21-year-old male. Four years ago, coalescent erythematous papules and reticular pigmentation on the chest, neck and the upper arms were observed. Histopathologic findings of erythematous papule showed moderate acanthosis, a few necrotic keratinocytes and focal hydropic degeneration in the basal cell layer in association with melanophages and perivascular lymphocytic infiltration of the upper dermis. The skin biopsy from the latest hyperpigmented lesion showed only melanophages and mild perivascular lymphocytic infiltrates in the upper dermis. During the 4 years of follow-up period, we noticed that reticulated hyperpigmentation is localized to the almost same sites with the previous papular lesion.
Subject(s)
Humans , Male , Young Adult , Arm , Biopsy , Dermis , Follow-Up Studies , Hyperpigmentation , Japan , Keratinocytes , Korea , Neck , Pigmentation , Prurigo , Skin , Thorax , Western WorldABSTRACT
Prurigo pigmentosa is a peculiar pruritic pigmented dermatosis characterized by the sudden appearance of reddish papules accompanied by severe pruritus. The etiology still remains unknown but environmental factors may play a role, in which physical trauma or friction from wet clothing induces the skin lesions. It has a seasonal preference for spring to summer when there is increased sweating. We report a case of prurigo pigmentosa which may be triggered by sweating and cured with minocycline.
Subject(s)
Clothing , Friction , Minocycline , Prurigo , Pruritus , Seasons , Skin , Skin Diseases , Sweat , SweatingABSTRACT
Prurigo pigmentosa is a rare dermatosis with unknown etiology. In some cases, systemic conditons, such as diabetes mellitus, pregnancy, and fasting were associated with prurigo pigmentosa. We report a typical case of prurigo pigmentosa associated with pregnancy in a 29-year-old female. When she was first pregnant at the age of 27, the skin lesions developed and disappeared after delivery without treatment . When the skin lesions recurred at the age of 29, we treated her with dapsone, 100 mg daily, which resulted in almost regression of the skin lesion. When she became pregnant again at the age of 30, the skin lesions recurred. After delivery of the second baby, the skin lesions disappeared rapidly without treatment.
Subject(s)
Adult , Female , Humans , Pregnancy , Dapsone , Diabetes Mellitus , Fasting , Prurigo , Skin , Skin DiseasesABSTRACT
Prurigo pigmentosa is a distinct type of inflammatory dermatosis characterized by recurrent pruritic erythematous papules that resolve, leaving a peculiar, reticulate hyperpigmentation. Although the cause of prurigo pigmentosa is unknown, exogenous factors, such as friction from clothing, have been suggested as possible triggers in some cases. In other cases, however, systemic conditions such as diabetes mellitus, pregnancy, fasting, and dieting were associated with prurigo pigmentosa, suggesting involvement of endogenous factors in the pathogenesis. There have been reported cases of prurigo pigmentosa associated with ketosis. Because ketosis is commonly observed in association with fasting, dieting, and insulin dependent diabetes melitus, it may be involved in the pathogenesis of prurigo pigmentosa. Here, we describe a patient with prurigo pigmentosa associated with diabetes mellitus and ketosis. In this case, the prurigo pigmentosa improved with the disappearance of the ketosis, but the blood sugar levels did not correlate with the improvement.
Subject(s)
Humans , Pregnancy , Blood Glucose , Clothing , Diabetes Mellitus , Diet , Fasting , Friction , Hyperpigmentation , Insulin , Ketosis , Prurigo , Skin DiseasesABSTRACT
Prurigo pigmentosa(PP) is a rare dermatosis with an unknown cause characterized by intensely pruritic papules that resolve with reticulate pigmentation. It is a disease entitity commonly reported in Japan but seemingly rare in other parts of the world. We report 5 cases that clinically and histopathologically fulfilled the characterisitics of PP and were treated with dapsone resulting in significant clinical improvement.